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Anomalous ependyma inducing split cord and meningomyelocele?
H P Flitman1, J Stanek, H W Hsu
1Department of Pathology and Laboratory Medicine, University of Cincinnati College of Medicine, Ohio, USA.
Journal of Child Neurology
|November 26, 1999
Summary
This study details severe central nervous system defects in a fetus, including spinal cord duplication and brain abnormalities, likely caused by early ependymal development issues.
Area of Science:
- Developmental neuroscience
- Fetal medicine
- Teratology
Background:
- Understanding early embryonic development is crucial for identifying causes of congenital abnormalities.
- The ependyma plays a key role in neural development and tissue induction.
Observation:
- A 17-18 week female fetus presented with significant central nervous system malformations.
- Observed were thoracic vertebral rachischisis with segmental diplomyelia (duplicated spinal cords) and an open neural tube defect (meningomyelocele).
- Brain anomalies included heterotopic ependymal cells near the choroid plexus and cerebellar heterotopia.
Findings:
- The duplicated spinal cords were unequal in size, within a single sheath without a septum.
- An unsplit segment of the neural tube exposed the ependyma, indicative of a meningomyelocele.
- Proliferating heterotopic ependymal cells suggest abnormal ependymal development and migration.
Implications:
- A common pathogenic theme of problematic ependymal development and migration may underlie these complex defects.
- The causative agent likely acted during the second week postconception, though it remains unidentified.
- This case highlights the critical role of early ependymal cell function in normal central nervous system formation.